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Merck
CN

95187

Supelco

Methylmalonyl-L-carnitine lithium salt

analytical standard

别名:

(2R)-3-Carboxy-2-(2-carboxy-1-oxopropoxy)-N,N,N-trimethyl-1-propanaminium inner salt lithium salt, O-Methylmalonyl-L-carnitine lithium salt

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About This Item

经验公式(希尔记法):
C11H19NO6 · xLi+
分子量:
261.27 (free acid basis)
UNSPSC代码:
41116107

等级

analytical standard

质量水平

检测方案

≥90.0% (HPLC)

形式

solid

旋光性

[α]/D -33.0±2.0°, c = 0.1 in H2O

保质期

limited shelf life, expiry date on the label

应用

clinical testing

储存温度

2-8°C

SMILES字符串

C[N+](C)(C)C[C@H](OC(C(C)C(O)=O)=O)CC([O-])=O

生化/生理作用

O-Methylmalonyl-L-carnitine belongs to the class of acyl carnitines. Acyl carnitines contain a fatty acid with the carboxylic acid attached to carnitine through an ester bond. The level of methylmalonyl carnitine has been found to be increased in patients with methylmalonuric aciduria (MMA), one of the most frequent organic acidurias caused by enzymatic defects involved in the catabolism of branched-chain amino acids.

WGK

WGK 3

闪点(°F)

Not applicable

闪点(°C)

Not applicable


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William R Wikoff et al.
Clinical chemistry, 53(12), 2169-2176 (2007-10-24)
We applied untargeted mass spectrometry-based metabolomics to the diseases methylmalonic acidemia (MMA) and propionic acidemia (PA). We used a screening platform that used untargeted, mass-based metabolomics of methanol-extracted plasma to find significantly different molecular features in human plasma samples from
Cristiano Rizzo et al.
Clinica chimica acta; international journal of clinical chemistry, 429, 30-33 (2013-11-26)
Methylmalonic aciduria (MMA) is one of the most frequent organic acidurias, a class of diseases caused by enzymatic defects mainly involved in the catabolism of branched-chain amino acids. Recently, mild MMA and C4-dicarboxylyl-carnitine (C4DC-C) accumulation have been reported in patients

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